Published in Brain on June 23, 2003
A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes. Proc Natl Acad Sci U S A (2005) 2.47
Prion strain discrimination in cell culture: the cell panel assay. Proc Natl Acad Sci U S A (2007) 2.30
Isolation from cattle of a prion strain distinct from that causing bovine spongiform encephalopathy. PLoS Pathog (2006) 1.55
Prion protein glycosylation is not required for strain-specific neurotropism. J Virol (2009) 1.31
Prions can infect primary cultured neurons and astrocytes and promote neuronal cell death. Proc Natl Acad Sci U S A (2004) 1.27
Prominent and persistent extraneural infection in human PrP transgenic mice infected with variant CJD. PLoS One (2008) 1.24
Endogenous proteolytic cleavage of disease-associated prion protein to produce C2 fragments is strongly cell- and tissue-dependent. J Biol Chem (2010) 1.14
PrPc does not mediate internalization of PrPSc but is required at an early stage for de novo prion infection of Rov cells. J Virol (2007) 1.01
Propagation of RML prions in mice expressing PrP devoid of GPI anchor leads to formation of a novel, stable prion strain. PLoS Pathog (2012) 0.98
Selective amplification of classical and atypical prions using modified protein misfolding cyclic amplification. J Biol Chem (2012) 0.96
Evidence for distinct chronic wasting disease (CWD) strains in experimental CWD in ferrets. J Gen Virol (2011) 0.94
Regulating factors of PrP glycosylation in Creutzfeldt-Jakob disease--implications for the dissemination and the diagnosis of human prion strains. PLoS One (2008) 0.92
Glycan-controlled epitopes of prion protein include a major determinant of susceptibility to sheep scrapie. J Virol (2004) 0.92
Sialylation of prion protein controls the rate of prion amplification, the cross-species barrier, the ratio of PrPSc glycoform and prion infectivity. PLoS Pathog (2014) 0.90
Functional stability of unliganded envelope glycoprotein spikes among isolates of human immunodeficiency virus type 1 (HIV-1). PLoS One (2011) 0.90
Prion propagation in cells expressing PrP glycosylation mutants. J Virol (2011) 0.88
Glycosylphosphatidylinositol anchor analogues sequester cholesterol and reduce prion formation. J Biol Chem (2010) 0.87
Glycosylation-related genes are variably expressed depending on the differentiation state of a bioaminergic neuronal cell line: implication for the cellular prion protein. Glycoconj J (2008) 0.85
Prion protein (PrP) gene-knockout cell lines: insight into functions of the PrP. Front Cell Dev Biol (2015) 0.85
Amyloid-β and proinflammatory cytokines utilize a prion protein-dependent pathway to activate NADPH oxidase and induce cofilin-actin rods in hippocampal neurons. PLoS One (2014) 0.85
N-glycans and glycosylphosphatidylinositol-anchor act on polarized sorting of mouse PrP(C) in Madin-Darby canine kidney cells. PLoS One (2011) 0.85
Prion infection of epithelial Rov cells is a polarized event. J Virol (2004) 0.84
Loss of Cellular Sialidases Does Not Affect the Sialylation Status of the Prion Protein but Increases the Amounts of Its Proteolytic Fragment C1. PLoS One (2015) 0.83
Generation of human scFvs antibodies recognizing a prion protein epitope expressed on the surface of human lymphoblastoid cells. BMC Biotechnol (2007) 0.82
PrP(Sc)-specific antibodies with the ability to immunodetect prion oligomers. PLoS One (2011) 0.82
Insoluble cellular prion protein and its association with prion and Alzheimer diseases. Prion (2011) 0.82
The glycosylation status of PrPC is a key factor in determining transmissible spongiform encephalopathy transmission between species. J Virol (2015) 0.81
Prion protein-specific antibodies that detect multiple TSE agents with high sensitivity. PLoS One (2014) 0.81
Differential Toxicity of Antibodies to the Prion Protein. PLoS Pathog (2016) 0.80
A camelid anti-PrP antibody abrogates PrP replication in prion-permissive neuroblastoma cell lines. PLoS One (2010) 0.79
Atypical and classical forms of the disease-associated state of the prion protein exhibit distinct neuronal tropism, deposition patterns, and lesion profiles. Am J Pathol (2013) 0.79
Docosahexaenoic and eicosapentaenoic acids increase prion formation in neuronal cells. BMC Biol (2008) 0.79
Disease-associated prion protein elicits immunoglobulin M responses in vivo. Mol Med (2006) 0.79
Species and strain glycosylation patterns of PrPSc. PLoS One (2009) 0.78
Glycoform-independent prion conversion by highly efficient, cell-based, protein misfolding cyclic amplification. Sci Rep (2016) 0.78
Mutated but Not Deleted Ovine PrPC N-Terminal Polybasic Region Strongly Interferes with Prion Propagation in Transgenic Mice. J Virol (2015) 0.77
Divergent prion strain evolution driven by PrP(C) expression level in transgenic mice. Nat Commun (2017) 0.75
Cross-seeding of prions by aggregated α-synuclein leads to transmissible spongiform encephalopathy. PLoS Pathog (2017) 0.75
Genome-wide association study identifies variants at CLU and PICALM associated with Alzheimer's disease. Nat Genet (2009) 15.15
TREM2 variants in Alzheimer's disease. N Engl J Med (2012) 11.35
Common variants at ABCA7, MS4A6A/MS4A4E, EPHA1, CD33 and CD2AP are associated with Alzheimer's disease. Nat Genet (2011) 9.23
Fluvastatin for prevention of cardiac events following successful first percutaneous coronary intervention: a randomized controlled trial. JAMA (2002) 4.94
Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis. Science (2003) 4.57
Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia. Nat Genet (2005) 4.21
Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics. Science (2003) 3.59
The Princeton III Consensus recommendations for the management of erectile dysfunction and cardiovascular disease. Mayo Clin Proc (2012) 3.45
Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report. Lancet (2006) 3.12
BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J (2002) 2.98
Guidelines on the diagnosis and management of solitary plasmacytoma of bone and solitary extramedullary plasmacytoma. Br J Haematol (2004) 2.91
Human prion protein with valine 129 prevents expression of variant CJD phenotype. Science (2004) 2.82
Prion propagation and toxicity in vivo occur in two distinct mechanistic phases. Nature (2011) 2.74
Frontotemporal dementia with the C9ORF72 hexanucleotide repeat expansion: clinical, neuroanatomical and neuropathological features. Brain (2012) 2.65
Early and late benefits of high-dose atorvastatin in patients with acute coronary syndromes: results from the PROVE IT-TIMI 22 trial. J Am Coll Cardiol (2005) 2.62
Detection of prion infection in variant Creutzfeldt-Jakob disease: a blood-based assay. Lancet (2011) 2.60
Targeting cellular prion protein reverses early cognitive deficits and neurophysiological dysfunction in prion-infected mice. Neuron (2007) 2.58
Genetic evidence implicates the immune system and cholesterol metabolism in the aetiology of Alzheimer's disease. PLoS One (2010) 2.57
Autoantibodies in sporadic Creutzfeldt-Jakob disease. JAMA Neurol (2013) 2.42
Molecular classification of sporadic Creutzfeldt-Jakob disease. Brain (2003) 2.42
Guidelines on the diagnosis and management of solitary plasmacytoma of bone and solitary extramedullary plasmacytoma. Clin Oncol (R Coll Radiol) (2004) 2.35
Monoclonal antibodies inhibit prion replication and delay the development of prion disease. Nature (2003) 2.31
FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration. Acta Neuropathol (2010) 2.30
A systematic review of prion therapeutics in experimental models. Brain (2006) 2.25
Real time quaking-induced conversion analysis of cerebrospinal fluid in sporadic Creutzfeldt-Jakob disease. Ann Neurol (2012) 2.18
A distinct clinical, neuropsychological and radiological phenotype is associated with progranulin gene mutations in a large UK series. Brain (2008) 2.13
Large C9orf72 hexanucleotide repeat expansions are seen in multiple neurodegenerative syndromes and are more frequent than expected in the UK population. Am J Hum Genet (2013) 2.09
Burkitt's lymphoma. Lancet (2012) 2.02
Disease-associated prion protein oligomers inhibit the 26S proteasome. Mol Cell (2007) 2.01
Analysis of 2000 consecutive UK tonsillectomy specimens for disease-related prion protein. Lancet (2004) 1.96
Interaction between prion protein and toxic amyloid β assemblies can be therapeutically targeted at multiple sites. Nat Commun (2011) 1.96
Genetic risk factors for variant Creutzfeldt-Jakob disease: a genome-wide association study. Lancet Neurol (2009) 1.94
Amyloid beta-protein dimers rapidly form stable synaptotoxic protofibrils. J Neurosci (2010) 1.92
Variant CJD in an individual heterozygous for PRNP codon 129. Lancet (2009) 1.84
Sexual dysfunction and cardiac risk (the Second Princeton Consensus Conference). Am J Cardiol (2005) 1.83
Detection and characterization of proteinase K-sensitive disease-related prion protein with thermolysin. Biochem J (2008) 1.77
Prevalence of disease related prion protein in anonymous tonsil specimens in Britain: cross sectional opportunistic survey. BMJ (2009) 1.75
Time course of the interaction between tadalafil and nitrates. J Am Coll Cardiol (2003) 1.71
Single treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease. Proc Natl Acad Sci U S A (2008) 1.71
Contribution of problem drug users' deaths to excess mortality in Scotland: secondary analysis of cohort study. BMJ (2008) 1.69
A novel prion disease associated with diarrhea and autonomic neuropathy. N Engl J Med (2013) 1.69
Prognostic utility of erectile dysfunction for cardiovascular disease in younger men and those with diabetes. Am Heart J (2012) 1.65
Low testosterone associated with obesity and the metabolic syndrome contributes to sexual dysfunction and cardiovascular disease risk in men with type 2 diabetes. Diabetes Care (2011) 1.60
Evidence based medicine inevitably increases testosterone prescribing. BMJ (2012) 1.54
A novel protective prion protein variant that colocalizes with kuru exposure. N Engl J Med (2009) 1.53
Treatment, risk factors, and outcome of adults with relapsed AML after reduced intensity conditioning for allogeneic stem cell transplantation. Blood (2011) 1.50
Update on human prion disease. Biochim Biophys Acta (2007) 1.46
Genetic associations with thalidomide mediated venous thrombotic events in myeloma identified using targeted genotyping. Blood (2008) 1.45
Inherited prion disease with 4-octapeptide repeat insertion: disease requires the interaction of multiple genetic risk factors. Brain (2011) 1.45
Molecular and clinical classification of human prion disease. Br Med Bull (2003) 1.45
Diagnosis and management of acute graft-versus-host disease. Br J Haematol (2012) 1.43
Summary of the recommendations on sexual dysfunctions in men. J Sex Med (2010) 1.42
Disease-related prion protein forms aggresomes in neuronal cells leading to caspase activation and apoptosis. J Biol Chem (2005) 1.42
Homozygosity for the C9orf72 GGGGCC repeat expansion in frontotemporal dementia. Acta Neuropathol (2013) 1.41
Less than half of patients aged 65 years or under with myeloma proceed to transplantation: results of a two region population-based survey. Br J Haematol (2005) 1.41
Sensitive and specific detection of sporadic Creutzfeldt-Jakob disease brain prion protein using real-time quaking-induced conversion. J Gen Virol (2011) 1.40
CEMO in a UK stallion. Vet Rec (2002) 1.39
Giving up smoking. Hosp Med (2004) 1.39
Believe-it then PROVE-IT. Int J Clin Pract (2004) 1.39
Atypical scrapie prions from sheep and lack of disease in transgenic mice overexpressing human prion protein. Emerg Infect Dis (2013) 1.38
Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice. Proc Natl Acad Sci U S A (2008) 1.37
Subclinical prion infection. Trends Microbiol (2003) 1.36
Disruption of endocytic trafficking in frontotemporal dementia with CHMP2B mutations. Hum Mol Genet (2010) 1.35
Ligation of TLR9 induced on human IL-10-secreting Tregs by 1alpha,25-dihydroxyvitamin D3 abrogates regulatory function. J Clin Invest (2009) 1.35
The role of CHMP2B in frontotemporal dementia. Biochem Soc Trans (2009) 1.34
Phenotypic heterogeneity in inherited prion disease (P102L) is associated with differential propagation of protease-resistant wild-type and mutant prion protein. Brain (2006) 1.34
Identification of genetic loci affecting mouse-adapted bovine spongiform encephalopathy incubation time in mice. Neurogenetics (2002) 1.31
Distinct glycoform ratios of protease resistant prion protein associated with PRNP point mutations. Brain (2006) 1.31
Evidence evaluation: a response to the court of appeal judgment in R v T. Sci Justice (2011) 1.30
Subclinical prion infection in humans and animals. Br Med Bull (2003) 1.25
Superoxide dismutase 1 and tgSOD1 mouse spinal cord seed fibrils, suggesting a propagative cell death mechanism in amyotrophic lateral sclerosis. PLoS One (2010) 1.25
Characterization of two distinct prion strains derived from bovine spongiform encephalopathy transmissions to inbred mice. J Gen Virol (2004) 1.23
Dissociation of pathological and molecular phenotype of variant Creutzfeldt-Jakob disease in transgenic human prion protein 129 heterozygous mice. Proc Natl Acad Sci U S A (2006) 1.22
Interpreting small quantities of DNA: the hierarchy of propositions and the use of Bayesian networks. J Forensic Sci (2002) 1.20
Parietal lobe deficits in frontotemporal lobar degeneration caused by a mutation in the progranulin gene. Arch Neurol (2008) 1.20
CHMP2B C-truncating mutations in frontotemporal lobar degeneration are associated with an aberrant endosomal phenotype in vitro. Hum Mol Genet (2007) 1.20
Suicide trends and geographical variations in the United Kingdom, 1991-2004. Health Stat Q (2006) 1.19
Chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein. J Gen Virol (2010) 1.19
HECTD2 is associated with susceptibility to mouse and human prion disease. PLoS Genet (2009) 1.18
Rational targeting for prion therapeutics. Nat Rev Neurosci (2005) 1.18
Mapping the progression of progranulin-associated frontotemporal lobar degeneration. Nat Clin Pract Neurol (2008) 1.17
Absence of spontaneous disease and comparative prion susceptibility of transgenic mice expressing mutant human prion proteins. J Gen Virol (2009) 1.16
Genetic variability in CLU and its association with Alzheimer's disease. PLoS One (2010) 1.16
Interaction between the phosphodiesterase 5 inhibitor, tadalafil and 2 alpha-blockers, doxazosin and tamsulosin in healthy normotensive men. J Urol (2004) 1.16
Inherited prion disease with six octapeptide repeat insertional mutation--molecular analysis of phenotypic heterogeneity. Brain (2006) 1.15
The residue 129 polymorphism in human prion protein does not confer susceptibility to Creutzfeldt-Jakob disease by altering the structure or global stability of PrPC. J Biol Chem (2004) 1.14
An enzyme-detergent method for effective prion decontamination of surgical steel. J Gen Virol (2005) 1.14
Misfolded PrP impairs the UPS by interaction with the 20S proteasome and inhibition of substrate entry. EMBO J (2011) 1.13
Molecular diagnosis of human prion disease. Methods Mol Biol (2008) 1.12
Genome-wide association study in multiple human prion diseases suggests genetic risk factors additional to PRNP. Hum Mol Genet (2011) 1.12
Trends and geographical variations in alcohol-related deaths in the United Kingdom, 1991-2004. Health Stat Q (2007) 1.12
Genetics of prion disease. Top Curr Chem (2011) 1.11